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Caroline Hayhurst, Dawn Williams, Jawad Yousaf, David Richardson, Barry Pizer, and Conor Mallucci

Object

Skull base tumors in children are rare but require complex approaches with potential morbidity to the developing craniofacial skeleton, in addition to tumor-related morbidity. Reports of long-term clinical and functional outcome following skull base approaches in children are scarce. The authors report long-term outcome in children with tumors undergoing multidisciplinary skull base surgery.

Methods

A retrospective analysis was undertaken of children undergoing surgery at a single institution between 1998 and 2008 for benign and malignant lesions of the anterior, middle, or posterior cranial base. Patients with craniopharyngioma, pituitary tumors, and optic glioma were excluded. Histology, surgical morbidity, length of hospital stay, progression-free survival, and adjuvant therapy were recorded. Functional and cognitive outcome was assessed prospectively using the Late Effects Severity Score (LESS).

Results

Twenty-three children ranging in age from 13 months to 15 years underwent skull base approaches for resection of tumors during the study period. The median follow-up duration was 60 months. Tumor types included meningioma, schwannoma, rhabdomyosarcoma, neuroblastoma, angiofibroma, and chordoma. Complete resection was achieved in 12 patients (52%). Thirteen patients (57%) had benign histology. The median hospital stay was 7 days. There were 3 deaths, 1 perioperative and 2 from tumor progression. Two patients had CSF leakage (9%) and 2 developed meningitis. Two children (9%) had residual neurological deficit at last follow-up evaluation. Thirteen (59%) of 22 surviving patients received adjuvant therapy. The majority of the patients remain in mainstream education and 19 of the 20 surviving children have an LESS of 3 or lower.

Conclusions

Children tolerate complex skull base procedures well, with minimal surgical-related morbidity as well as good long-term tumor control rates and functional outcomes from maximal safe resection combined with adjuvant treatment when required.

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Donncha F. O’Brien, Caroline Hayhurst, Barry Pizer, and Conor L. Mallucci

Object

The primary aim of this study was to evaluate the success of endoscopic third ventriculostomy (ETV) as a treatment for obstructive hydrocephalus secondary to midline tumors (midbrain, pontine, pineal, tectal plate, thalamic, and third ventricular regions). In addition, the study examined the role and value of endoscopic tumor biopsy (ETB) in the management of such cases. All surgical procedures were performed through a single-trajectory approach.

Methods

A retrospective analysis of clinical notes, operation records, and pre- and postventriculostomy neuroimaging data was performed to determine the success or failure and complications of ETV and ETB in 42 patients presenting with tumor-induced obstructive hydrocephalus. Patient data were derived from an endoscopy database initiated in 1998. The study population included 21 female and 21 male patients (mean age 37 years, range 5–77 years). All 42 patients underwent an ETV; 33 of the 42 underwent an ETV and an ETB (single-trajectory). One patient was excluded from the follow-up analysis due to rapid deterioration of his condition from tumor progression. The duration of follow up ranged from 3 to 84 months (mean 32 months).

At the last follow up, 11 patients with ETVs had undergone shunt placement and two patients had undergone repeated ETVs, giving a long-term success rate of 68% (28 of 41 cases) for single ETV as a treatment for hydrocephalus at presentation. Statistical analysis revealed no significant relationship (p > 0.92) between tumor location and ETV success or failure. The mean time to ETV failure was 32 weeks. Histological examination of biopsy specimens was nondiagnostic in eight (24%) of the 33 cases in which ETB was performed. Seven of these cases involved pineal region tumors and one involved a tectal plate tumor. There was no death or major morbidity associated with ETV and ETB in this series.

Conclusions

Endoscopic third ventriculostomy is a safe and durable means of controlling hydrocephalus in tumor cases. Its success rate is high—comparable to that reported in aqueduct stenosis cases. Although ETB is probably not as accurate for diagnosis as biopsy with frame-based stereotactic guidance, it is associated with a lower mortality rate and, in the correct clinical setting, may be justifiably attempted as an initial biopsy procedure at the same time as ETV via a single-trajectory approach.

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John Goodden, Barry Pizer, Benedetta Pettorini, Dawn Williams, Jo Blair, Mohammed Didi, Nicky Thorp, and Conor Mallucci

Object

Optic pathway/hypothalamic gliomas (OPHGs) are generally benign tumors situated in an exquisitely sensitive brain region. The location and natural history of OPHGs has led to much debate about optimal treatment. This paper revisits the role of and optimal timing of debulking surgery in OPHG.

Methods

This paper presents a series of cases managed by the neuro-oncology team at Alder Hey Children's Hospital and a single surgeon. Data were collected retrospectively for periods prior to 2009 and prospectively thereafter. Tailored treatment strategies were used, including observation and combinations of surgery, chemotherapy, and radiotherapy. Tumor control rates and outcomes are reviewed.

Results

Forty-two patients were treated between 1998 and 2011. Their median age at diagnosis was 5 years 7 months. Nineteen patients were positive for neurofibromatosis Type 1 (NF1) and 23 patients were negative for NF1. The median duration of follow-up was 77 months (range 21.8–142.3 months). Presenting symptoms included visual impairment (in 50% of cases), headache (in 24%), and hypothalamic/pituitary dysfunction (in 29%).

Twenty-two debulking procedures were performed in 21 patients. Four biopsies (3 open, 1 endoscopic) were also performed. The histological diagnosis was pilocytic astrocytoma in 21 patients and pilomyxoid astrocytoma in 2 patients. Ten patients (Group 1) had primary surgical debulking alone and were then observed. Four patients (Group 2) had surgical debulking, plus planned chemotherapy within 3 months. Seven patients (Group 3) required surgical debulking for progressive disease following a variety of treatments. Patient age had the greatest impact on subsequent tumor progression.

In total, 13 patients received chemotherapy, 4 on initial presentation, 4 in combination with surgery, and 5 for further tumor progression. Five patients were treated with radiotherapy, 3 prior to referral to Alder Hey.

Eleven patients required shunt insertion for hydrocephalus. Vision was stabilized for 74% of patients. The number of patients with hypothalamic/pituitary dysfunction increased from 12 at presentation to 16 by the end of treatment. The overall survival rate was 93%. Three patients died—1 from tumor progression, 1 from infective complications from tumor biopsy, and 1 from a spontaneous posterior fossa hemorrhage. NF1 was associated with improved outcome—fewer patients required active intervention and rates of visual impairment and/or or hypothalamic/pituitary dysfunction were lower.

Conclusions

Good long-term survival and functional outcomes can be achieved in children with OPHG. Tumor control was achieved through an individualized approach using surgery, chemotherapy, or radiotherapy in varied combinations. The authors aim to limit radiotherapy to cases involving older children in whom other therapies have failed, due to the well-described and often devastating late effects associated with midline cranial irradiation. Surgery has a clear role for diagnosis, tumor control, and relief of mass effect. In particular, primary surgical debulking of tumor (without adjuvant therapy) is safe and effective. Recent advances in intraoperative MRI may add value and need further assessment.

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Barry L. Pizer, Timothy Moss, Anthony Oakhill, David Webb, and Hugh B. Coakham

✓ Astroblastoma is a rare type of glial tumor, usually occurring in older children and young adults. It has a distinctive histological appearance that is characterized by a radiating arrangement of tumor cells that form perivascular pseudorosettes. The authors report only the second case of astroblastoma presenting in congenital form. Following subtotal tumor resection, the infant received 10 courses of chemotherapy consisting of vincristine, etoposide, and carboplatinum. Evidence is presented for a tumor response to chemotherapy, a previously unreported observation. The child is alive 2.5 years after diagnosis with satisfactory functional status. Immunohistological and ultrastructural features of this tumor are presented. The discussion focuses on the biology, natural history, and management of this unusual neoplasm.

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Mitchell T. Foster, Dawn Hennigan, Rebecca Grayston, Kirsten van Baarsen, Geraint Sunderland, Christopher Paul Millward, Harishchandra Lalgudi Srinivasan, Deborah Ferguson, Teddy Totimeh, Barry Pizer, and Conor Mallucci

OBJECTIVE

Complications in pediatric neurooncology surgery are seldom and inconsistently reported. This study quantifies surgical morbidity after pediatric brain tumor surgery from the last decade in a single center, using existing morbidity and outcome measures.

METHODS

The authors identified all pediatric patients undergoing surgery for an intracranial tumor in a single tertiary pediatric neurosurgery center between January 2008 and December 2018. Complications between postoperative days 0 and 30 that had been recorded prospectively were graded using appropriate existing morbidity scales, i.e., the Clavien-Dindo (CD), Landriel, and Drake scales. The result of surgery with respect to the predetermined surgical aim was also recorded.

RESULTS

There were 477 cases (364 craniotomies and 113 biopsies) performed on 335 patients (188 males, median age 9 years). The overall 30-day mortality rate was 1.26% (n = 6), and no deaths were a direct result of surgical complication. Morbidity on the CD scale was 0 in 55.14%, 1 in 10.69%, 2 in 18.66%, 3A in 1.47%, 3B in 11.74%, and 4 in 1.05% of cases. Morbidity using the Drake classification was observed in 139 cases (29.14%). Neurological deficit that remained at 30 days was noted in 8.39%; 78% of the returns to the operative theater were for CSF diversion.

CONCLUSIONS

To the authors’ knowledge, this is the largest series presenting outcomes and morbidity from pediatric brain tumor surgery. The mortality rate and morbidity on the Drake classification were comparable to those of published series. An improved tool to quantify morbidity from pediatric neurooncology surgery is necessary.

Free access

Elizabeth Schepke, Magnus Tisell, Colin Kennedy, Stephanie Puget, Paolo Ferroli, Mathilde Chevignard, François Doz, Barry Pizer, Stefan Rutkowski, Maura Massimino, Aurora Navajas, Edward Schwalbe, Debbie Hicks, Steven C. Clifford, Torsten Pietsch, and Birgitta Lannering

OBJECTIVE

Extensive resection of a tumor in the posterior fossa in children is associated with the risk of neurological deficits. The objective of this study was to prospectively evaluate the short-term neurological morbidity in children after medulloblastoma surgery and relate this to the tumor’s growth pattern and to the extent of resection.

METHODS

In 160 patients taking part in the HIT-SIOP PNET 4 (Hyperfractionated Versus Conventionally Fractionated Radiotherapy in Standard Risk Medulloblastoma) trial, neurosurgeons prospectively responded to questions concerning the growth pattern of the tumor they had resected. The extent of resection (gross, near, or subtotal) was evaluated using MRI. The patients’ neurological status before resection and around 30 days after resection was recorded.

RESULTS

Invasive tumor growth, defined as local invasion in the brain or meninges, cranial nerve, or major vessel, was reported in 58% of the patients. After surgery almost 70% of all patients were affected by one or several neurological impairments (e.g., impaired vision, impaired extraocular movements, and ataxia). However, this figure was very similar to the preoperative findings. Invasive tumor growth implied a significantly higher number of impairments after surgery (p = 0.03) and greater deterioration regarding extraocular movements (p = 0.012), facial weakness (p = 0.048), and ataxia in the arms (p = 0.014) and trunk (p = 0.025) compared with noninvasive tumor growth. This deterioration was not dependent on the extent of resection performed. Progression-free survival (PFS) at 5 years was 80% ± 4% and 76% ± 5% for patients with invasive and noninvasive tumor growth, respectively, with no difference in the 5-year PFS for extent of resection.

CONCLUSIONS

Preoperative neurological impairments and invasive tumor growth were strong predictors of deterioration in short-term neurological outcome after medulloblastoma neurosurgery, whereas the extent of resection was not. Neither tumor invasiveness nor extent of resection influenced PFS. These findings support the continuation of maximal safe resection in medulloblastoma surgery where functional risks are not taken in areas with tumor invasion.